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1.
Acta Neurochir (Wien) ; 165(1): 265-269, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-35934751

RESUMO

Epileptic seizure is the common symptom associated with lipomas in the Sylvian fissure (Sylvian lipomas). Removal of these lipomas carries risks of hemorrhage and brain damage. We report a surgical strategy of not removing the lipoma in a case of intractable temporal lobe epilepsy associated with Sylvian lipoma. We performed anterior temporal lobectomy with preservation of the pia mater of the Sylvian fissure and achieved seizure freedom. Focal cortical dysplasia type 1 of the epileptic neocortex adjacent to the Sylvian lipoma was pathologically diagnosed. We recommend our surgical procedure in similar cases to avoid complications and achieve adequate seizure control.


Assuntos
Neoplasias Encefálicas , Epilepsia do Lobo Temporal , Epilepsia , Lipoma , Humanos , Epilepsia do Lobo Temporal/diagnóstico por imagem , Epilepsia do Lobo Temporal/etiologia , Epilepsia do Lobo Temporal/cirurgia , Imageamento por Ressonância Magnética/efeitos adversos , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Convulsões , Lipoma/complicações , Lipoma/diagnóstico por imagem , Lipoma/cirurgia
2.
Inorg Chem ; 60(3): 1550-1560, 2021 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-33241691

RESUMO

Two different four-electron reductions of dioxygen (O2) on a metal surface are reproduced in homogeneous systems. The reaction of the highly unsaturated (56-electron) tetraruthenium tetrahydride complex 1 with O2 readily afforded the bis(µ3-oxo) complex 3 via a dissociative mechanism that includes large electronic and geometric changes, i.e., a four-electron oxidation of the metal centers and an increase of 8 in the number of valence electrons. In contrast, the tetraruthenium hexahydride complex 2 induces a smooth H-atom transfer to the incorporated O2 species, and the O-OH bond is cleaved to afford the mono(µ3-oxo) complex 4 via an associative mechanism. Density functional theory calculations suggest that the higher degree of unsaturation in the tetrahydride system induces a significant interaction between the tetraruthenium core and the O2 moiety, enabling the large changes required for the dissociative mechanism.

3.
Epilepsy Behav ; 102: 106688, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31805503

RESUMO

OBJECTIVE: Postictal generalized electroencephalographic suppression (PGES) has been associated with sudden unexpected death in epilepsy (SUDEP) in adults. Decreased heart rate variability (HRV) is one clinical marker of SUDEP in adults with epilepsy. The objective of this study was to analyze the characteristics of HRV associated with generalized convulsive seizures (GCS) ±â€¯PGES in children. METHODS: Nine hundred and seventy-seven consecutive children who underwent prolonged scalp video-EEG (vEEG) and 1-lead electrocardiogram (ECG) monitoring at the Hospital for Sick Children, Toronto, Ontario, Canada were reviewed retrospectively from 2009 to 2011. Thirty-five children had GCS captured during their vEEG with or without PGES and met inclusion criteria. Children were subdivided into three age groups and compared with age-matched controls: 3-6 years; 7-12 years; and 13-18 years. Interictal HRV was measured at 5 min during N2 sleep. Preictal HRV was measured at 1 h prior to GCS onset, and postictal HRV was measured at 3 min post-GCS cessation. Low frequency (LF: ms2, 0.04-0.15 Hz) and high frequency (HF: ms2, 0.15-0.4 Hz) bands of heart rate oscillations were analyzed during the interictal and preictal periods. The root mean square of successive differences (RMSSDs) was analyzed during the following time points: interictal; preictal; and postictal. RESULTS: Thirty-five children had GCS: 18 children with PGES [3-6 years (n = 2); 7-12 years (n = 6); 13-18 years (n = 10)] and 17 children without PGES [3-6 years (n = 6); 7-12 years (n = 5); 13-18 years (n = 6)]. Seventeen additional age-matched controls were identified [3-6 years (n = 3); 7-12 years (n = 5); 13-18 years (n = 9)]. Seventy-four GCS were captured consisting of 36 GCS + PGES and 38 GCS - PGES. There was no difference of interictal HRV among children with GCS ±â€¯PGES and controls. The preictal LF and HF in 36 GCS + PGES were significantly higher compared with 38 GCS - PGES (p < 0.01). The postictal RMSSD in 36 GCS + PGES was significantly higher compared with 38 GCS - PGES (p < 0.01). The pre- to postictal RMSSD change was significantly lower in children with GCS + PGES than in those with GCS - PGES (p = 0.035). CONCLUSIONS: In summary, the preictal HRV in GCS + PGES was significantly higher than in children with GCS - PGES. The higher remaining postictal RMSSD in children with GCS + PGES is a potential indicator of autonomic dysregulation. In certain children with epilepsy, autonomic dysregulation may contribute to poor recovery from a GCS with subsequent PGES, thereby contributing to SUDEP. Heart rate variability and autonomic regulation in children with epilepsy should be further studied prospectively in order to better understand the mechanism by which PGES may lead to SUDEP.


Assuntos
Eletroencefalografia/métodos , Epilepsia Generalizada/fisiopatologia , Frequência Cardíaca/fisiologia , Convulsões/fisiopatologia , Morte Súbita Inesperada na Epilepsia , Adulto , Criança , Eletroencefalografia/tendências , Epilepsia Generalizada/epidemiologia , Feminino , Humanos , Masculino , Ontário/epidemiologia , Estudos Retrospectivos , Convulsões/epidemiologia , Fases do Sono/fisiologia , Morte Súbita Inesperada na Epilepsia/epidemiologia
4.
J Neuroradiol ; 46(4): 268-275, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30853545

RESUMO

Quantitative magnetic resonance imaging (MRI) with multislice, multi-echo, and multi-delay acquisition enables simultaneous quantification of R1 and R2 relaxation rates, proton density, and the B1 field in a single acquisition, and requires only about 6 minutes for full-head coverage. Using dedicated SyMRI software, radiologists can generate any contrast-weighted image by manipulating the acquisition parameters, including repetition time, echo time, and inversion time. Moreover, automatic brain tissue segmentation, volumetry, and myelin measurement can also be performed. Using the SyMRI approach, a shorter scan time, an objective examination, and personalized MR imaging parameters can be obtained in daily clinical pediatric imaging. Here we summarize and review the use of SyMRI in imaging of the pediatric brain, including the basic principles of MR quantification along with its features, clinical applications, and limitations.


Assuntos
Encéfalo/diagnóstico por imagem , Processamento de Imagem Assistida por Computador/métodos , Imageamento por Ressonância Magnética , Encéfalo/anatomia & histologia , Encéfalo/patologia , Encefalopatias/diagnóstico por imagem , Encefalopatias/patologia , Criança , Humanos , Bainha de Mielina/patologia , Razão Sinal-Ruído , Software
5.
Inorg Chem ; 56(16): 9802-9813, 2017 Aug 21.
Artigo em Inglês | MEDLINE | ID: mdl-28783320

RESUMO

The trinuclear zirconium imido complex [{LZr(NMe2)}3] (2, L = C5Me4CH2CH2N) was synthesized by amine elimination between Zr(NMe2)4 and endo-olefinic isomers of (tetramethylcyclopentadienyl)ethanamine (LH3) (1). To study the fundamental reactivity of the trizirconium system, reactions of 2 with primary amines were examined. Selective incorporations of the primary amines were observed, depending on steric and electronic natures of the amine substrates. The amine-incorporated complexes [(LZrNHR)(LHZrNHR)(LHZr)(µ-NHR)(µ3-NR)] (3, R = Pr, Et), [(LZrNHR)2(LHZr)(µ-NR)] (4, R = Pr, i-Bu), [(LZr)2(LZrNMe2)(µ-NR)] (5, R = neo-Pen), and [(LZr)(LZrNHAr)(LH2Zr)(µ-NAr)2] (6, Ar = Ph, C6H4-4-Br, C6H4-4-OMe) were structurally characterized by NMR and XRD analysis and showed several coordination modes of the substrate nitrogen ligands: i.e., terminal amides, bridging amides, and bridging imides but not terminal imides. Thermolysis of a mixture of 3 and 4 led to C-H bond activation, giving rise to the zirconaaziridines [{LZr(η2-NCHR)}(LZr)(LHZr)(µ-NHCH2R)] (12, R = Et, Me). Complex 2 proved to be a competent precatalyst in the hydroamination of the aminoalkynes (H2NCH2CR12CH2C≡CR2) (13, R1 = H, R2 = Bu, Ph, t-Bu; 14, R1 = Me, R2 = Et, Ph). Stoichiometric or semicatalytic reactions of 2 and the aminoalkynes were studied to explore the reactivity of in situ formed Zr3 species.

6.
Angew Chem Int Ed Engl ; 54(49): 14871-4, 2015 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-26487526

RESUMO

A µ3-η(2):η(2):η(2)-silane complex, [(Cp*Ru)3(µ3-η(2):η(2):η(2)-H3SitBu)(µ-H)3] (2 a; Cp* = η(5)-C5Me5), was synthesized from the reaction of [{Cp*Ru(µ-H)}3(µ3-H)2] (1) with tBuSiH3. Complex 2 a is the first example of a silane ligand adopting a µ3-η(2):η(2):η(2) coordination mode. This unprecedented coordination mode was established by NMR and IR spectroscopy as well as X-ray diffraction analysis and supported by a density functional study. Variable-temperature NMR analysis implied that 2 a equilibrates with a tautomeric µ3-silyl complex (3 a). Although 3 a was not isolated, the corresponding µ3-silyl complex, [(Cp*Ru)3(µ3-η(2):η(2)-H2SiPh)(H)(µ-H)3] (3 b), was obtained from the reaction of 1 with PhSiH3. Treatment of 2 a with PhSiH3 resulted in a silane exchange reaction, leading to the formation of 3 b accompanied by the elimination of tBuSiH3. This result indicates that the µ3-silane complex can be regarded as an "arrested" intermediate for the oxidative addition/reductive elimination of a primary silane to a trinuclear site.

7.
Inorg Chem ; 53(13): 6634-54, 2014 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-24915499

RESUMO

Zr-Ir hydrido complexes with ansa-(cyclopentadienyl)(amide) as the supporting ligand in the zirconium fragment, e.g., (L(1)ZrR)(Cp*Ir)(µ-H)3 [L(1) = Me2Si(η(5)-C5Me4)(N(t)Bu), R = Cl (5), Ph (7), Me (10), alkyl, and aryl] were designed, synthesized, and isolated as tractable early-late heterodinuclear complexes. Despite the presence of the three supporting hydride ligands, Zr-Ir distances in the crystal structures of 5, alkyl, and aryl complexes [2.74-2.76 Å] were slightly longer than the sum of the element radii of Zr and Ir [2.719 Å]. These hydrocarbyl complexes displayed the thermolytic C-H activation of a variety of aromatic compounds and several organometallic compounds. Also, the substrate scope and limitation in the Zr-Ir system were studied. The regiochemical outcomes during the C-H activation of pyridine derivatives and methoxyarenes suggested the in situ generation of a Lewis acidic active intermediate, i.e., (L(1)Zr)(Cp*IrH2) (III). The existence of III and relevant σ-complex intermediates {L(1)Zr(η(2)-R-H)}(Cp*IrH2) (IIR) (R = Me, Ph) in the ligand exchange was demonstrated by the direct isolation of a Et3PO-adduct of III (39b) from 7 and kinetic studies. The structure of the direct Zr-Ir bonds in IIPh, IIMe, III, and 39b were probed using computational studies. The unprecedented strong M-M' interactions in the early-late heterobimetallic (ELHB) complexes have been proposed herein.

8.
Inorg Chem ; 53(10): 5100-8, 2014 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-24801527

RESUMO

We investigated herein the reactions of (Me3tacn)FeCln (1a: n = 3, 1b: n = 2) with common aluminum hydride reagents and a bulky dihydridoaluminate {Li(ether)2}{Al(OC6H3-2,6-(t)Bu2)}(µ-H)2, which yielded the diamagnetic hydrido complexes 2-4 containing Fe(II) and Al(III). In particular, the use of divalent 1b afforded excellent isolated yields. The structures of 2-4 were determined using spectroscopic and crystallographic analyses. The crystal structures showed distorted octahedral Fe centers and fairly short Fe-Al distances [2.19-2.24 Å]. The structures of cation moiety 2 and neutral complex 4 were further probed using DFT calculations, which indicated a stable low-spin Fe(II) state and strongly electron-donating nature of the (Me3tacn)FeH3 fragment toward the Al(III) center.


Assuntos
Alumínio/química , Compostos Aza/química , Complexos de Coordenação/síntese química , Ferro/química , Complexos de Coordenação/química , Modelos Moleculares , Estrutura Molecular , Teoria Quântica
9.
IEEE Trans Biomed Eng ; 71(2): 531-541, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37624716

RESUMO

Temporallobe epilepsy (TLE) has been conceptualized as a brain network disease, which generates brain connectivity dynamics within and beyond the temporal lobe structures in seizures. The hippocampus is a representative epileptogenic focus in TLE. Understanding the causal connectivity in terms of brain network during seizures is crucial in revealing the triggering mechanism of epileptic seizures originating from the hippocampus (HPC) spread to the lateral temporal cortex (LTC) by ictal electrocorticogram (ECoG), particularly in high-frequency oscillations (HFOs) bands. In this study, we proposed the unified-epoch dynamic causality analysis method to investigate the causal influence dynamics between two brain regions (HPC and LTC) at interictal and ictal phases in the frequency range of 1-500 Hz by introducing the phase transfer entropy (PTE) out/in-ratio and sliding window. We also proposed PTE-based machine learning algorithms to identify epileptogenic zone (EZ). Nine patients with a total of 26 seizures were included in this study. We hypothesized that: 1) HPC is the focus with the stronger causal connectivity than that in LTC in the ictal state at gamma and HFOs bands. 2) Causal connectivity in the ictal phase shows significant changes compared to that in the interictal phase. 3) The PTE out/in-ratio in the HFOs band can identify the EZ with the best prediction performance.


Assuntos
Epilepsia do Lobo Temporal , Epilepsia , Humanos , Epilepsia do Lobo Temporal/diagnóstico por imagem , Entropia , Eletrocorticografia/métodos , Convulsões , Eletroencefalografia
10.
Sci Rep ; 14(1): 11491, 2024 05 20.
Artigo em Inglês | MEDLINE | ID: mdl-38769115

RESUMO

Several attempts for speech brain-computer interfacing (BCI) have been made to decode phonemes, sub-words, words, or sentences using invasive measurements, such as the electrocorticogram (ECoG), during auditory speech perception, overt speech, or imagined (covert) speech. Decoding sentences from covert speech is a challenging task. Sixteen epilepsy patients with intracranially implanted electrodes participated in this study, and ECoGs were recorded during overt speech and covert speech of eight Japanese sentences, each consisting of three tokens. In particular, Transformer neural network model was applied to decode text sentences from covert speech, which was trained using ECoGs obtained during overt speech. We first examined the proposed Transformer model using the same task for training and testing, and then evaluated the model's performance when trained with overt task for decoding covert speech. The Transformer model trained on covert speech achieved an average token error rate (TER) of 46.6% for decoding covert speech, whereas the model trained on overt speech achieved a TER of 46.3% ( p > 0.05 ; d = 0.07 ) . Therefore, the challenge of collecting training data for covert speech can be addressed using overt speech. The performance of covert speech can improve by employing several overt speeches.


Assuntos
Interfaces Cérebro-Computador , Eletrocorticografia , Fala , Humanos , Feminino , Masculino , Adulto , Fala/fisiologia , Percepção da Fala/fisiologia , Adulto Jovem , Estudos de Viabilidade , Epilepsia/fisiopatologia , Redes Neurais de Computação , Pessoa de Meia-Idade , Adolescente
11.
J Neural Eng ; 21(3)2024 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-38648781

RESUMO

Objective.Invasive brain-computer interfaces (BCIs) are promising communication devices for severely paralyzed patients. Recent advances in intracranial electroencephalography (iEEG) coupled with natural language processing have enhanced communication speed and accuracy. It should be noted that such a speech BCI uses signals from the motor cortex. However, BCIs based on motor cortical activities may experience signal deterioration in users with motor cortical degenerative diseases such as amyotrophic lateral sclerosis. An alternative approach to using iEEG of the motor cortex is necessary to support patients with such conditions.Approach. In this study, a multimodal embedding of text and images was used to decode visual semantic information from iEEG signals of the visual cortex to generate text and images. We used contrastive language-image pretraining (CLIP) embedding to represent images presented to 17 patients implanted with electrodes in the occipital and temporal cortices. A CLIP image vector was inferred from the high-γpower of the iEEG signals recorded while viewing the images.Main results.Text was generated by CLIPCAP from the inferred CLIP vector with better-than-chance accuracy. Then, an image was created from the generated text using StableDiffusion with significant accuracy.Significance.The text and images generated from iEEG through the CLIP embedding vector can be used for improved communication.


Assuntos
Interfaces Cérebro-Computador , Eletrocorticografia , Humanos , Masculino , Feminino , Eletrocorticografia/métodos , Adulto , Eletroencefalografia/métodos , Pessoa de Meia-Idade , Eletrodos Implantados , Adulto Jovem , Estimulação Luminosa/métodos
12.
Clin Neurophysiol ; 148: 44-51, 2023 04.
Artigo em Inglês | MEDLINE | ID: mdl-36796285

RESUMO

OBJECTIVE: To analyze chronological changes in phase-amplitude coupling (PAC) and verify whether PAC analysis can diagnose epileptogenic zones during seizures. METHODS: We analyzed 30 seizures in 10 patients with mesial temporal lobe epilepsy who had ictal discharges with preictal spiking followed by low-voltage fast activity patterns on intracranial electroencephalography. We used the amplitude of two high-frequency bands (ripples: 80-200 Hz, fast ripples: 200-300 Hz) and the phase of three slow wave bands (0.5-1 Hz, 3-4 Hz, and 4-8 Hz) for modulation index (MI) calculation from 2 minutes before seizure onset to seizure termination. We evaluated the accuracy of epileptogenic zone detection by MI, in which a combination of MI was better for diagnosis and analyzed patterns of chronological changes in MI during seizures. RESULTS: MIRipples/3-4 Hz and MIRipples/4-8 Hz in the hippocampus were significantly higher than those in the peripheral regions from seizure onset. Corresponding to the phase on intracranial electroencephalography, MIRipples/3-4 Hz decreased once and subsequently increased again. MIRipples/4-8 Hz showed continuously high values. CONCLUSIONS: Continuous measurement of MIRipples/3-4 Hz and MIRipples/4-8 Hz could help identify epileptogenic zones. SIGNIFICANCE: PAC analysis of ictal epileptic discharges can help epileptogenic zone identification.


Assuntos
Epilepsia do Lobo Temporal , Humanos , Epilepsia do Lobo Temporal/diagnóstico , Eletroencefalografia , Convulsões/diagnóstico , Eletrocorticografia , Hipocampo
13.
Pediatr Neurol ; 143: 6-12, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-36934517

RESUMO

BACKGROUND: Hemispherectomy is an optimal treatment for patients with Sturge-Weber syndrome (SWS) affecting the whole hemisphere; however, a consensus has not been reached regarding therapeutic choices for those with involvement of two to three lobes. In this study, we compared seizure and cognitive outcomes between medical and surgical treatment groups in patients with multilobar involvement. METHODS: We evaluated 50 patients with multilobar involvement. Surgical indications included (1) antiepileptic drug (AED)-resistant seizures; (2) developmental delay; and (3) cortical atrophy. Twenty-nine patients were classified in the medical treatment group (MTG), and 21 patients were in the surgical treatment group (STG). Seizure type and frequency, SWS electroencephalography score (SWS-EEGS), and pretherapeutic and posttherapeutic SWS neurological scores (SWS-NS) were compared between groups. Median ages at the initial evaluation of the MTG and STG were 4 and 2 years, and at the final evaluation were 13 and 17 years, respectively. RESULTS: The STG had a higher incidence (76.2%) of focal to bilateral tonic-clonic seizures and status epilepticus, although no difference in SWS-EEGS. Seizure and cognitive subcategories of SWS-NS at initial evaluation were worse in the STG (P = 0.025 and P = 0.007). The seizure subcategory in MTG and STG improved after therapy (P = 0.002 and P = 0.001). Cognition was maintained in MTG and improved in STG (P = 0.002). The seizure-free rates in MTG and STG were 58.6% and 85.7%, respectively. CONCLUSIONS: Appropriate therapeutic choices improved seizure outcomes. Although patients who required surgery had more severe epilepsy and cognitive impairment, surgery improved both.


Assuntos
Epilepsia , Hemisferectomia , Síndrome de Sturge-Weber , Humanos , Síndrome de Sturge-Weber/complicações , Síndrome de Sturge-Weber/cirurgia , Epilepsia/tratamento farmacológico , Epilepsia/etiologia , Epilepsia/cirurgia , Convulsões/etiologia , Cognição , Hemisferectomia/efeitos adversos
14.
Front Neurol ; 14: 1258854, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37780707

RESUMO

Objective: Vagus nerve stimulation (VNS) is a palliative surgery for drug-resistant epilepsy. The two objectives of this study were to (1) determine the seizure type most responsive to VNS and (2) investigate the preventive effect on status epilepticus (SE) recurrence. Methods: We retrospectively reviewed 136 patients with drug-resistant epilepsy who underwent VNS implantation. We examined seizure outcomes at 6, 12, and 24 months following implantation of VNS as well as at the last visit to the Juntendo Epilepsy Center. Univariate analysis and multivariate logistic regression models were used to estimate the prognostic factors. Results: 125 patients were followed up for at least 1 year after VNS implantation. The percentage of patients with at least a 50% reduction in seizure frequency compared with prior to VNS implantation increased over time at 6, 12, and 24 months after VNS implantation: 28, 41, and 52%, respectively. Regarding overall seizure outcomes, 70 (56%) patients responded to VNS. Of the 40 patients with a history of SE prior to VNS implantation, 27 (67%) showed no recurrence of SE. The duration of epilepsy, history of SE prior to VNS implantation and seizure type were correlated with seizure outcomes after VNS implantation in univariate analysis (p = 0.05, p < 0.01, and p = 0.03, respectively). In multivariate logistic regression analysis, generalized seizure was associated with VNS response [odds ratio (OR): 4.18, 95% CI: 1.13-15.5, p = 0.03]. A history of SE prior to VNS implantation was associated with VNS non-responders [(OR): 0.221, 95% CI: 0.097-0.503, p < 0.01]. The duration of epilepsy, focal to bilateral tonic-clonic seizure and epileptic spasms were not significantly associated with VNS responders (p = 0.07, p = 0.71, and p = 0.11, respectively). Conclusion: Following 125 patients with drug-resistant epilepsy for an average of 69 months, 56% showed at least 50% reduction in seizure frequency after VNS implantation. This study suggests that generalized seizure is the most responsive to VNS, and that VNS may reduce the risk of recurrence of SE. VNS was shown to be effective against generalized seizure and also may potentially influence the risk of further events of SE, two marker of disease treatment that can lead to improved quality of life.

15.
Acta Neuropathol Commun ; 11(1): 33, 2023 03 02.
Artigo em Inglês | MEDLINE | ID: mdl-36864519

RESUMO

Focal cortical dysplasia is the most common malformation during cortical development, sometimes excised by epilepsy surgery and often caused by somatic variants of the mTOR pathway genes. In this study, we performed a genetic analysis of epileptogenic brain malformed lesions from 64 patients with focal cortical dysplasia, hemimegalencephy, brain tumors, or hippocampal sclerosis. Targeted sequencing, whole-exome sequencing, and single nucleotide polymorphism microarray detected four germline and 35 somatic variants, comprising three copy number variants and 36 single nucleotide variants and indels in 37 patients. One of the somatic variants in focal cortical dysplasia type IIB was an in-frame deletion in MTOR, in which only gain-of-function missense variants have been reported. In focal cortical dysplasia type I, somatic variants of MAP2K1 and PTPN11 involved in the RAS/MAPK pathway were detected. The in-frame deletions of MTOR and MAP2K1 in this study resulted in the activation of the mTOR pathway in transiently transfected cells. In addition, the PTPN11 missense variant tended to elongate activation of the mTOR or RAS/MAPK pathway, depending on culture conditions. We demonstrate that epileptogenic brain malformed lesions except for focal cortical dysplasia type II arose from somatic variants of diverse genes but were eventually linked to the mTOR pathway.


Assuntos
Neoplasias Encefálicas , Displasia Cortical Focal , Malformações do Desenvolvimento Cortical do Grupo I , Malformações do Sistema Nervoso , Humanos , Malformações do Desenvolvimento Cortical do Grupo I/genética , Encéfalo
16.
Seizure ; 94: 23-25, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-34837729

RESUMO

PURPOSE: The removal of the bottom of sulcus dysplasia (BOSD) often includes the gyral crown; however, this method has been controversial. We hypothesized that the epileptogenic zone of the BOSD does not include the gyral crown. To reveal the depth and extent of the epileptogenic zone of the BOSD, we applied the two electrophysiological modalities: (1) the occurrence rate (OR) of high-frequency oscillations (HFOs) and (2) modulation index (MI), reflecting the strength of phase-amplitude coupling between HFOs and slow oscillations. METHODS: We investigated the ripples [80-200 Hz] and fast ripples [200-300 Hz]) in HFOs and MI (HFOs [80-300 Hz] and slow oscillations [3-4 Hz]). We opened the sulcus at the BOSD and implanted the subdural electrodes directly over the MRI visible lesion. All patients (n = 3) underwent lesionectomy and the gyral crown was preserved. RESULTS: Pathological findings demonstrated focal cortical dysplasia type IIb and seizure freedom was achieved. The OR of the HFOs was not significantly different between the BOSD and the gyral crown. In contrast, the MI between HFOs and slow oscillations in the BOSD was significantly higher than that in the gyral crown. CONCLUSION: High MI values distinguished the epileptogenic BOSD from the non-epileptogenic gyral crowns. MI could be a more informative biomarker of epileptogenicity than the OR of HFOs in a subset of patients with the BOSD.


Assuntos
Epilepsia , Malformações do Desenvolvimento Cortical do Grupo I , Humanos , Imageamento por Ressonância Magnética
17.
Seizure ; 100: 1-7, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35687962

RESUMO

OBJECTIVE: We assessed the diagnostic utility of the occurrence rate of high-frequency oscillations and modulation index (MI) from intraoperative electrocorticography (ioECoG) in determining the extent of epileptogenicity in mesial temporal lobe epilepsy (TLE) with hippocampal sclerosis (HS). METHODS: We enrolled 17 patients who underwent selective amygdalohippocampectomy (SelAH) for TLE due to HS. We analyzed the occurrence rate of ripples (80-200 Hz) and fast ripples (200-300 Hz); and MI between ripples and 3-4 Hz (MIRipples/3-4 Hz) and fast ripples and 3-4 Hz (MIFRs/3-4 Hz) from the amygdala, hippocampus, and lateral temporal lobe (LTL) pre-SelAH and the LTL post-SelAH, and subsequently categorized the patients into good and poor seizure outcome groups. We compared the occurrence rates and MIs over each region of interest between both groups. Receiver operating characteristic analysis was used to identify the most optimal indicator to predict poor surgical outcomes. RESULTS: In the poor seizure outcome group, an increase in the occurrence rate of ripples was seen in the hippocampus and LTL pre-SelAH and the LTL post-SelAH. The MIRipples/3-4 Hz from the LTL pre-SelAH was the most indicative factor of poor outcome. CONCLUSIONS: High occurrence rate of ripples and MIRipples/3-4 Hz from the LTL showed wide epileptogenicity in TLE patients with poor seizure outcomes after SelAH. Our data suggest that the analysis of the occurrence rate of HFOs and MIHFOs/3-4 Hz from ioECoG, especially from the LTL, can indicate the distribution of epileptogenicity in TLE with HS.


Assuntos
Epilepsia do Lobo Temporal , Doenças Neurodegenerativas , Eletrocorticografia , Eletroencefalografia , Epilepsia do Lobo Temporal/complicações , Epilepsia do Lobo Temporal/cirurgia , Hipocampo/cirurgia , Humanos , Esclerose , Convulsões
18.
Brain Dev ; 44(6): 410-414, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35393130

RESUMO

We describe a case of severe encephalopathy with reversible splenial lesion associated with parechovirus, followed by intractable temporal lobe epilepsy (TLE), which was improved by epilepsy surgery. A 3-year-old girl was admitted because of fever, consciousness disturbance and generalized tonic clonic seizure. Her seizure lasted for four hours. Fluid-attenuated inversion recovery (FLAIR) showed a hyperintensity in the splenium of the corpus callosum. Electroencephalogram (EEG) demonstarated continuous diffuse epileptic activity represented by synchronous and rhythmic high-amplitude spikes and waves, which led to the diagnosis of status epilepticus. Her consciousness was improved with fosphenytoin, midazolam and methylprednisolone pulse after 3 days. Seven days later, FLAIR hyperintensity in the splenium of the corpus callosum was disappeared; however, a hyperintensity in the right hippocampus was detected. Since the stool examination was positive for parechovirus, her final diagnosis was reversible splenial lesion syndrome (RESLES) associated with parechovirus. At age 8, she experienced epigastric sensation and consciousness disturbance once a week. Based on the scalp EEG and radiological findings, she was diagnosed with intractable right TLE. We performed a right selective amygdalohippocampectomy and anterior temporal disconnection at 10 years of age. One year and 3 months after surgery, she was seizure free. To our knowledge, this is the first report of severe febrile epilepticus status. with RESLES associated with parechovirus, followed by intractable TLE, which was resolved by epilepsy surgery.


Assuntos
Encefalopatias , Epilepsia Resistente a Medicamentos , Encefalite , Epilepsia , Parechovirus , Estado Epiléptico , Encefalopatias/patologia , Criança , Pré-Escolar , Corpo Caloso/patologia , Corpo Caloso/cirurgia , Epilepsia Resistente a Medicamentos/complicações , Epilepsia Resistente a Medicamentos/cirurgia , Encefalite/complicações , Epilepsia/complicações , Feminino , Febre/complicações , Hipocampo/diagnóstico por imagem , Hipocampo/patologia , Hipocampo/cirurgia , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Convulsões/etiologia , Estado Epiléptico/complicações , Estado Epiléptico/cirurgia , Síndrome
19.
Gan To Kagaku Ryoho ; 38(2): 313-5, 2011 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-21368503

RESUMO

Cetuximab, a monoclonal antibody used to target the epidermal growth factor receptor(EGFR), was approved for refractory and metastatic colon cancer expressing EGFR, and the EGFR expression must be confirmed. But the EGFR expression may become false negative by immunohistochemistry. When we used a past operation specimen for a search in particular, a correct evaluation may be difficult for prolonged formalin fixation. We report a case successfully treated by cetuximab, who was diagnosed as EGFR-negative by the past operation specimen, but as EGFR-positive by the liver biopsy specimen. A 51- year old woman with multiple organ metastases, who had experienced failure with prior oxaliplatin, irinotecan(CPT-11), 5- FU and bevacizumab regimens, was administered cetuximab plus CPT-11 because it was EGFR-positive by liver biopsy, and the tumor was obviously reduced. It is useful to obtain another specimen such as by liver biopsy, when the EGFR expression is negative by a past operation specimen.


Assuntos
Anticorpos Monoclonais/uso terapêutico , Neoplasias do Colo/tratamento farmacológico , Receptores ErbB/metabolismo , Neoplasias Hepáticas/tratamento farmacológico , Anticorpos Monoclonais Humanizados , Biópsia , Cetuximab , Neoplasias do Colo/metabolismo , Neoplasias do Colo/patologia , Feminino , Humanos , Neoplasias Hepáticas/metabolismo , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/secundário , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Tomografia Computadorizada por Raios X
20.
J Neurosurg ; : 1-8, 2021 Dec 03.
Artigo em Inglês | MEDLINE | ID: mdl-34861650

RESUMO

OBJECTIVE: Tailored surgery to extensively resect epileptogenic lesions using intraoperative electrocorticography (ioECoG) may improve seizure outcomes. However, resection of large areas is associated with decreased memory function postoperatively. The authors assessed whether ioECoG could provide useful information on how to minimize the focus resection and obtain better seizure outcomes without memory deterioration. They examined the postoperative seizure-free period and memory alteration in a retrospective cohort of patients with mesial temporal lobe epilepsy (TLE) due to hippocampal sclerosis (HS) in whom the extent of removal was determined using ioECoG findings. METHODS: The authors enrolled 82 patients with TLE associated with HS who were treated surgically. Transsylvian amygdalohippocampectomy was indicated as the first step. When visual inspection identified interictal epileptic discharges from the lateral temporal lobe on ioECoG, anterior temporal lobectomy (ATL) was eventually performed. The patients were divided into the selective amygdalohippocampectomy (SA, n = 40) and ATL (n = 42) groups. Postoperative seizure outcomes were assessed at 1, 2, 3, 5, and 7 years postoperatively using the International League Against Epilepsy classification. The Kaplan-Meier survival analysis was applied to evaluate the period of seizure recurrence between the SA and ATL groups. Factors attributed to seizure recurrence were analyzed using the Cox proportional hazards model, and they were as follows: epileptic focal laterality; age at seizure onset (< 10 or ≥ 10 years old); seizure frequency (more than weekly or less than weekly seizures); history of focal to bilateral tonic-clonic seizure; infectious etiology; and surgical procedure. The Wechsler Memory Scale-Revised was used to evaluate memory function pre- and postoperatively. RESULTS: Seizure outcomes were significantly worse in the SA group than in the ATL group at 2 years postoperatively (p = 0.045). The International League Against Epilepsy class 1 outcomes at 7 years postoperatively in the SA and ATL groups were 63% and 81%, respectively. Kaplan-Meier analysis showed that seizure recurred significantly earlier in the SA group than in the ATL group (p = 0.031). The 2-way ANOVA analysis was used to compare the SA and ATL groups in each memory category, and revealed that there was no significant difference regardless of the side of surgery. CONCLUSIONS: Visual assessment of ioECoG cannot be used as an indicator to minimize epileptic focus resection in patients with TLE associated with HS. ATL is more effective in obtaining seizure-free outcomes; however, both ATL and SA can preserve memory function.

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